CONCLUSIONS
Complex lymphatic anomalies can be debilitating, and complications of lymphatic leak can be life-threatening. Unfortunately, the field lacks basic natural history outcome trials and any prospective interventional trials dedicated to the treatment of symptomatic lymphatic anomalies or lymphatic leak. What is certain, however, is the need for a multidisciplinary, coordinated approach to the management of lymphatic leak. Management can be triaged based on the rate of lymphatic leak (low vs high flow) with aggressive nutritional support. Patience is mandatory as reduction in lymphatic flow can take weeks to achieve. Lymphatic imaging, surgical, and endolymphatic techniques continue to advance. The expanding identification of genetic variants that drive abnormal lymphatic disease has opened the door to new pharmacotherapy options to augment disease. With such advancements on many fronts coordinated prospective trials may be on the horizon.
Conflict of Interests: T.A.N. has consulted for Novartis phamaceuticals to develop consensus guidelines for PIK3CA-related overgrowth disorders. All additional authors have no financial/personal conflicts to disclose
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Figure 1: The lymphatic system is a unidirectional transport system to return fluid to the blood circulation 1a: Intestinal lymphatics drain from mesenteric nodes to the cysterna chyli located around the aortic hiatus of the diaphragm and ascends cephalad through the thoracic duct to empty into the blood circulation via the subclavian to brachiocephalic veins. Pulmonary lymphatics drain from paratracheal and bronchopulmonary lymph nodes to right and left thoracic ducts to similarly empty into subclavian to brachiocephalic veins. The illustration depicts select lymphatics of the left lung and small intestine in a healthy neonate (1) thoracic duct (2) left brachiocephalic vein and branches (3) inferior vena cava (4) cysterna chyli. 1b: The illustration depicts select abnormalities of the lymphatic system (5) idiopathic stenosis of the thoracic duct; results in impaired lymphatic drainage, increased trans-pleural filtration pressure (lymphatic hypertension), retrograde lymphatic flow and increased permeability that results in lymphatic leak (6) large region of pulmonary lymphangiomatosis (over proliferation of lymphatic vessels) and lymphangiectasia (inappropriately dilated lymphatic vessels) enveloping the ventral surface of the lung; results in failure of appropriate lymphatic drainage (7) chylothorax collecting in the left pleural cavity (8) chylous ascites collecting in peritoneum causing a distended abdomen.
Figure 2: 2a) Maximal intensity projection (MIP) coronal projection of intranodal DCMRL in patient with KLA showing massively dilated and tortuous thoracic duct (arrow) and bilateral pulmonary perfusion (arrowheads) as well as mediastinal perfusion. 2b)MIP coronal projection of IN-DCMRL in patient with an EPHB4 mutation and lymphatic conduction disorder showing dermal backflow (arrowheads) and no flow into the thorax. 2c) MIP coronal projection of intrahepatic DCMRL in the same patient showing peritoneal leak (arrow), retrograde mesenteric flow, and conduction up left sided paravertebral channels supplying the left sided posterior intercostal networks (arrowhead).
Figure 3: Management of a lymphatic leak